PCNSL: Extranodal lymphoma confined to CNS with absence of systemic disease at time of diagnosis
DLBCL: Extranodal DLBCL confined to CNS
Excluded lymphomas arising in dura, intravascular large B-cell, T-cell, NK-cell, secondary CNS lymphomas
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bödör C et al: Molecular subtypes and genomic profile of primary central nervous system lymphoma. J Neuropathol Exp Neurol. 79(2):176-83, 2020
Green K et al: Cancer, central nervous system lymphoma, 2020
Lin X et al: Atypical radiological findings of primary central nervous system lymphoma. Neuroradiology. 62(6): 669-76, 2020
Siegal T et al: Primary CNS lymphoma in the elderly: the challenge. Acta Haematol. 141(3):138-45, 2019
Lukas RV et al: Diagnostic evaluation in primary CNS lymphoma. Neurologist. 23(2):53-4, 2018
Grommes C et al: Primary CNS lymphoma. J Clin Oncol. 35(21):2410-8, 2017
Lu SS et al: Histogram analysis of apparent diffusion coefficient maps for differentiating primary CNS lymphomas from tumefactive demyelinating lesions. AJR Am J Roentgenol. 204(4):827-34, 2015
Matinella A et al: Neurological complications of HIV infection in pre-HAART and HAART era: a retrospective study. J Neurol. 262(5):1317-27, 2015
Yamasaki F et al: Magnetic resonance spectroscopy detection of high lipid levels in intraaxial tumors without central necrosis: a characteristic of malignant lymphoma. J Neurosurg. 122(6):1370-9, 2015
Kickingereder P et al: Evaluation of microvascular permeability with dynamic contrast-enhanced MRI for the differentiation of primary CNS lymphoma and glioblastoma: radiologic-pathologic correlation. AJNR Am J Neuroradiol. 35(8):1503-8, 2014
Mansour A et al: MR imaging features of intracranial primary CNS lymphoma in immune competent patients. Cancer Imaging. 14:22, 2014
Lee IH et al: Analysis of perfusion weighted image of CNS lymphoma. Eur J Radiol. 76(1):48-51, 2010
Angelov L et al: Blood-brain barrier disruption and intra-arterial methotrexate-based therapy for newly diagnosed primary CNS lymphoma: a multi-institutional experience. J Clin Oncol. 27(21):3503-9, 2009
Correa DD et al: Prospective cognitive follow-up in primary CNS lymphoma patients treated with chemotherapy and reduced-dose radiotherapy. J Neurooncol. 91(3):315-21, 2009
Imai H et al: Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma. Pathol Int. 59(7):431-7, 2009
Kim DS et al: Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology. 251(2):467-75, 2009
Liao W et al: Differentiation of primary central nervous system lymphoma and high-grade glioma with dynamic susceptibility contrast-enhanced perfusion magnetic resonance imaging. Acta Radiol. 50(2):217-25, 2009
Sierra del Rio M et al: Primary CNS lymphoma in immunocompetent patients. Oncologist. 14(5):526-39, 2009
Fischer L et al: Meningeal dissemination in primary CNS lymphoma: prospective evaluation of 282 patients. Neurology. 71(14):1102-8, 2008
Kleinschmidt-DeMasters BK et al: Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications. J Neuropathol Exp Neurol. 67(11):1103-11, 2008
Mohile NA et al: The utility of body FDG PET in staging primary central nervous system lymphoma. Neuro Oncol. 10(2):223-8, 2008
Toh CH et al: Primary cerebral lymphoma and glioblastoma multiforme: differences in diffusion characteristics evaluated with diffusion tensor imaging. AJNR Am J Neuroradiol. 29(3):471-5, 2008
Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Malignant lymphomas. IARC Press. 188-92, 2007
Abrey LE et al: Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol. 24(36):5711-5, 2006
Hunt MA et al: Distinguishing primary central nervous system lymphoma from other central nervous system diseases: a neurosurgical perspective on diagnostic dilemmas and approaches. Neurosurg Focus. 21(5):E3, 2006
Iwamoto FM et al: Primary dural lymphomas: a review. Neurosurg Focus. 21(5):E5, 2006
Stadnik TW et al: Diffusion-weighted MR imaging of intracerebral masses: comparison with conventional MR imaging and histologic findings. AJNR Am J Neuroradiol. 22(5):969-76, 2001
Bataille B et al: Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 92(2):261-6, 2000
Schlegel U et al: Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment. J Neurol Sci. 181(1-2): 1-12, 2000
Koeller KK et al: Primary central nervous system lymphoma: radiologic-pathologic correlation. Radiographics. 17(6): 1497-526, 1997
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Primary CNS lymphoma (PCNSL)
Diffuse large B-cell lymphoma (DLBCL)
Definitions
PCNSL: Extranodal lymphoma confined to CNS with absence of systemic disease at time of diagnosis
DLBCL: Extranodal DLBCL confined to CNS
Excluded lymphomas arising in dura, intravascular large B-cell, T-cell, NK-cell, secondary CNS lymphomas
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Bödör C et al: Molecular subtypes and genomic profile of primary central nervous system lymphoma. J Neuropathol Exp Neurol. 79(2):176-83, 2020
Green K et al: Cancer, central nervous system lymphoma, 2020
Lin X et al: Atypical radiological findings of primary central nervous system lymphoma. Neuroradiology. 62(6): 669-76, 2020
Siegal T et al: Primary CNS lymphoma in the elderly: the challenge. Acta Haematol. 141(3):138-45, 2019
Lukas RV et al: Diagnostic evaluation in primary CNS lymphoma. Neurologist. 23(2):53-4, 2018
Grommes C et al: Primary CNS lymphoma. J Clin Oncol. 35(21):2410-8, 2017
Lu SS et al: Histogram analysis of apparent diffusion coefficient maps for differentiating primary CNS lymphomas from tumefactive demyelinating lesions. AJR Am J Roentgenol. 204(4):827-34, 2015
Matinella A et al: Neurological complications of HIV infection in pre-HAART and HAART era: a retrospective study. J Neurol. 262(5):1317-27, 2015
Yamasaki F et al: Magnetic resonance spectroscopy detection of high lipid levels in intraaxial tumors without central necrosis: a characteristic of malignant lymphoma. J Neurosurg. 122(6):1370-9, 2015
Kickingereder P et al: Evaluation of microvascular permeability with dynamic contrast-enhanced MRI for the differentiation of primary CNS lymphoma and glioblastoma: radiologic-pathologic correlation. AJNR Am J Neuroradiol. 35(8):1503-8, 2014
Mansour A et al: MR imaging features of intracranial primary CNS lymphoma in immune competent patients. Cancer Imaging. 14:22, 2014
Lee IH et al: Analysis of perfusion weighted image of CNS lymphoma. Eur J Radiol. 76(1):48-51, 2010
Angelov L et al: Blood-brain barrier disruption and intra-arterial methotrexate-based therapy for newly diagnosed primary CNS lymphoma: a multi-institutional experience. J Clin Oncol. 27(21):3503-9, 2009
Correa DD et al: Prospective cognitive follow-up in primary CNS lymphoma patients treated with chemotherapy and reduced-dose radiotherapy. J Neurooncol. 91(3):315-21, 2009
Imai H et al: Comparative clinicopathological study of primary CNS diffuse large B-cell lymphoma and intravascular large B-cell lymphoma. Pathol Int. 59(7):431-7, 2009
Kim DS et al: Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology. 251(2):467-75, 2009
Liao W et al: Differentiation of primary central nervous system lymphoma and high-grade glioma with dynamic susceptibility contrast-enhanced perfusion magnetic resonance imaging. Acta Radiol. 50(2):217-25, 2009
Sierra del Rio M et al: Primary CNS lymphoma in immunocompetent patients. Oncologist. 14(5):526-39, 2009
Fischer L et al: Meningeal dissemination in primary CNS lymphoma: prospective evaluation of 282 patients. Neurology. 71(14):1102-8, 2008
Kleinschmidt-DeMasters BK et al: Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications. J Neuropathol Exp Neurol. 67(11):1103-11, 2008
Mohile NA et al: The utility of body FDG PET in staging primary central nervous system lymphoma. Neuro Oncol. 10(2):223-8, 2008
Toh CH et al: Primary cerebral lymphoma and glioblastoma multiforme: differences in diffusion characteristics evaluated with diffusion tensor imaging. AJNR Am J Neuroradiol. 29(3):471-5, 2008
Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Malignant lymphomas. IARC Press. 188-92, 2007
Abrey LE et al: Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol. 24(36):5711-5, 2006
Hunt MA et al: Distinguishing primary central nervous system lymphoma from other central nervous system diseases: a neurosurgical perspective on diagnostic dilemmas and approaches. Neurosurg Focus. 21(5):E3, 2006
Iwamoto FM et al: Primary dural lymphomas: a review. Neurosurg Focus. 21(5):E5, 2006
Stadnik TW et al: Diffusion-weighted MR imaging of intracerebral masses: comparison with conventional MR imaging and histologic findings. AJNR Am J Neuroradiol. 22(5):969-76, 2001
Bataille B et al: Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg. 92(2):261-6, 2000
Schlegel U et al: Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment. J Neurol Sci. 181(1-2): 1-12, 2000
Koeller KK et al: Primary central nervous system lymphoma: radiologic-pathologic correlation. Radiographics. 17(6): 1497-526, 1997
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